Expanded CTG repeats in myotonin protein kinase increase susceptibility to oxidative stress
作者:
Fusako Usuki,
Shoichi Ishiura,
期刊:
NeuroReport
(OVID Available online 1998)
卷期:
Volume 9,
issue 10
页码: 2291-2296
ISSN:0959-4965
年代: 1998
出版商: OVID
关键词: Antioxidants;Apoptosis;CTG repeats;Methylmercury;Myotonin protein kinase;Myotonic dystrophy;Oxidative stress
数据来源: OVID
摘要:
THE effect of oxidative stress on myogenic cells with expanded CTG repeats in the myotonin protein kinase (MtPK) gene was investigated using MtPK cDNA-transformants in order to investigate the disease process underlying myotonic dystrophy. We employed methylmercury as a model for reagents that produce reactive oxygen species (ROS). Mutant MtPK cDNA transformants containing 46 CTG repeats treated with 1 μM methylmercury for 24 h underwent cell death showing the characteristics of apoptosis. In contrast, methylmercury-induced cytotoxicity was weaker in wild type MtPK cDNA transformants. Antioxidants such as N-acetyl-L-cysteine and trolox suppressed methylmercury-induced apoptosis, indicating that the intracellular generation of ROS plays an important role. These studies suggest that expanded CTG repeats in MtPK increase the susceptibility of cells to oxidative stress.
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